Clinical Neuroscience

[Report]

LEEL-ŐSSY Lóránt

MARCH 20, 1994

Clinical Neuroscience - 1994;47(03-04)

[Report - International Joint Conference on Stroke and Cerebral Circulation January 7-10, 1994. Bombay, India.]

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Clinical Neuroscience

[Young neurologists XI. forum]

[Correlation between carotid artery lesions and cerebral perfusion (Carotid Duplex scan and brain SPECT). Stenosis or dissection? Differential diagnostic issues in carotid circulation disorders. AVM diagnosed by 3D TCD. Possible diagnostic errors in supraaortic duplex UH scans and angiography. Causes of diagnostic errors in duplex ultrasound studies of the carotid arteries and ways to avoid them. The role of duplex ultrasonography in the surgical evaluation of common carotid artery occlusion (case report). Follow-up of Moyamoya disease with transcranial Doppler (TCD). Platelet function tests in the acute and chronic phase of cerebral circulatory disease. Multicausal cerebral circulatory disorders. Co-occurrence of parkinsonism and giant aneurysm (case report). Teratoma adultum, germinoma, spinal and cerebral metastases in the background of intermittent headache - 5-year patient follow-up Differential diagnosis of meningeal tumours. Extracranial tumours presenting with symptoms of brain metastasis. Pitfalls in the diagnosis of craniocervical transitional and high cervical space occupying processes. Diagnosis of benign spinal tumours. Spinal dermoid cyst - data on the aetiology of low back pain syndrome. Experimental and clinical potential of brain microdialysis. Treatment of multiple intracranial tumours. Tremor and reflex tests in Parkinson's disease. HMPAO SPECT studies in Parkinson's disease. Aurorix treatment in cases of Parkinson's syndrome complicated with depression. Drug therapy of Parkinson's syndrome with special reference to diurnal performance fluctuations. Jumex in the early phase of Parkinson's syndrome. Conductive education in parkinsonism. Use of biophysical methods in the study of the pathomechanism of neurological diseases. Large blind spot syndrome. Investigation of optokinetic nystagmus in solitary frontal laesio. Hypocalcaemia and epilepsy. Difficulties in the recognition of epileptic seizures. Hypnosis treatment in partial epilepsy. Migraine, depression, anxiety. Verbalization features of headaches. Fahr's disease in our class material. CT scanning of cerebral vascular lesions and differential diagnostic difficulties. Binswanger's disease. CT lesions in patients with psychopathological symptoms. The importance of 3D MR angiography in occlusive cerebral vascular disease. Comparative study of cervical large vessel imaging. Difficulties in the diagnosis of craniocervical transition in a case report. Differential diagnosis of spinal cord disease. History and epidemiology of Parkinson's disease. Recent data on the pathomechanism of parkinsonism; experimental therapies Diagnostic errors in Parkinson's syndrome. Sinemet CR - advances in drug therapy. Oxidant phenotype studies in Parkinson's syndrome patients. Madopar-HBS treatment of patients with Parkinson's syndrome. Co-occurrence of complicated migraine and idiopathic cerebral atrophy. Family study in adreno-leuko-dystrophy. Electrophysiological study of patients with anaemia perniciosa. Cartilage drift into the sacral dura sac. Results of electrophysiological studies in patients with Parkinson's syndrome. EMG-SCAN studies in patients with Parkinson's syndrome. A case of adult myotubular myopathy in our department. Advanced picture of Kugelberg-Welander syndrome. Immunological study of idiopathic inflammatory myopathies. Recessive generalized myotonia (Becker). Differential diagnosis of myotonia in a case report. Glossopharyngeal neuralgia with syncope. Familial occurrence of multisystemic atrophy. Apert syndrome in the light of modern diagnostics. Infant with Reit syndrome (video case presentation). Juvenile cardiogenic stroke. Sneddon syndrome. Cerebrovascular patients in our department in the first half of 1993. Cerebellar haemorrhages. Changes in the assessment of prognosis in the patient population of our department. A case of medial thalamic atrophy thought to be multiple sclerosis. Extrapyramidal damage caused by stroke. Cases of bilateral occipital lobular atrophy. Case of severe brainstem lesion (central pontine myelinolysis). Long-term follow-up of the cellular and humoral immune response in patients with multiple sclerosis. Current problems of Lyme borreliosis in our departmental practice. Listeria monocytogenes as a possible causative agent of purulent meningitis. Incidence of lower limb root pains causing diagnostic difficulty in our department. Peripheral neuropathy in hypereosinophilic syndrome. Eye movement disorders caused by brain stem diseases. Depression in patients with Parkinson's syndrome, with particular reference to the possibility of presuicidal syndrome (RINGEL).]

Clinical Neuroscience

[Dementia and related problems '93. an overview]

TARISKA Péter

[Based on extensive investigations carried out the epidemiology of dementias in the last decade in Europe the methodology has become unified. The most important elements are: two-step screening in two time periods, population of 4000 or more, unified screening and diagnostic methods. The incidence was 1 p.c. established in the population between the ages of 60–64; the proportion nearly doubled in each 5 year period. Regarding the recently published risk factors of Alzheimer's disease (AD) the possible protective role of smoking and the low educational level seem to be very interesting. In the group of AAMI the biological life events as possible causative factors seem to be of importance. The functional assesment scale of Reisberg is reported from psychometric tests. An outline is given about the clinical diagnostic criteria of AD and vascular dementias based on the widely discussed system published on the latter in 1993. The problems of different clinical subtypes of AD with relevance to biochemical markers are discussed as are the diganostic criteria of Lewy body type dementia. A summary on some new etological results (genetic heterogenety, new possible ways of amyloidogenesis, glutamate-theory, etc.) is given. After highlihgting the importance of the different kinds of psychotherapy and mental training, social factors are stressed, and some ethical conflict situations (driving, coercive measures, etc.) are shortly presented.]

Clinical Neuroscience

[The history of Hungarian neurology (Part I)]

BEKÉNY György

[Every nation has its great people of whom it is proud. In our country, intellectuals think that Hungarians are particularly talented in music and mathematics. However, our outstanding geneticist believes that the genetic background of different abilities is equal between nations and that the different results are caused by external circumstances. Indeed, the "accumulation" of great Hungarian conductors and musicians in the United States, and the careers of many Nobel Prize-winning mathematicians and physicists in the United States, were made possible by the fact that they had to leave their homeland for political reasons. Contrary to what is suggested above, the first 50 years of a very successful and internationally respected period of Hungarian neuroscience were hampered by these circumstances. All the more reason to appreciate the neurological output of this period. I would be glad if the readers of this work would share this opinion. I have selected those publications for illustration which appeared in the so-called 'world languages'. The only exceptions are monographs, theses and one or two major works, some of which have already been published in a foreign language. Due to the limitations of space, many areas of neuroscience could not be discussed. Thus, I have not been able to write about international greats in neuroanatomy such as Mihály Lenhossék, Apáthy, Szentágothai; about neurophysiologists, especially those working on the cochleovestibular system such as Hőgyes, Bárány, Békésy; about neuroendocrinology or about many details of neurochemistry. The importance of neurosurgery, which is closely related to our subject, calls for a separate presentation. A small monograph on the history of Hungarian neuroscience was published in 1976 by István Környey, a great teacher and scholar of Hungarian neurology. In 1992, Zoltán Nagy published a history of Hungarian neurology in the last century under the title Hungarian Neurology in The Last Century. These historical summaries were important precursors to my present work. ]

Clinical Neuroscience

[Voltage mapping studies of generalized spike- wave patterns associated with absence seizures]

CLEMENS Béla

[In this retrospective study, scalp electric fields of ictal generalized spike-wave discharges were analized by the so-called topographic voltage mapping method. The 17 patients displaying absences (with or without other seizures) belonged to different age groups and diverse epileptic syndromes. Main results: 1. Maps derived at the points of the spike and the wave components belong to different classes. A-type spikes show frontal, P-type spikes show posterior voltage maxima. Also atypical spike (map) configurations exist. 2. A spike maps frequently show a characteristic modification along the GSW pattern. 3. Waves can display bilateral (L) or medial (M) frontal voltage maxima. 4. At least in the time window of several weeks, the dominant spike map pattern and the pattern of the waveform was characteristic to each patient. Configurations of the different spike and wave components show some relation to a limited set of clinical data. The combination of A- spikes with L-waves was found in children who had , typical" absence- epilepsies. On the contrary, irregular spectrum of different spikes and M-waves was found in elder absence patients showing rather unfavorable course of their illness.]

Clinical Neuroscience

[Immunological test for idiopathic inflammatory myopathies]

HORVÁTH Rita, ALZIRA A S Carvalmo

[Sceletal muscle biopsy specimens from patients with various inflammatory myopathies – dermatomyositis, polymyositis and inclusion body myositis – have been investigated by immunocytochemical methods with the help of monoclonal antibodies. Conclusions about the pathomechanism of these disorders were saught. In dermatomyositis the humoral immunity and the damage of the small vessels of muscle fibres may play an important role. The appearance of class I MHC antigens on diseased muscle may make the affected tissue a target for cytotoxic T8 cells, and may thus have a role in muscle fibre damage in polymyositis and inclusion body myositis.]

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Clinical Neuroscience

Late simultaneous carcinomatous meningitis, temporal bone infiltrating macro-metastasis and disseminated multi-organ micro-metastases presenting with mono-symptomatic vertigo – a clinico-pathological case reporT

JARABIN András János, KLIVÉNYI Péter, TISZLAVICZ László, MOLNÁR Anna Fiona, GION Katalin, FÖLDESI Imre, KISS Geza Jozsef, ROVÓ László, BELLA Zsolt

Although vertigo is one of the most common complaints, intracranial malignant tumors rarely cause sudden asymmetry between the tone of the vestibular peripheries masquerading as a peripheral-like disorder. Here we report a case of simultaneous temporal bone infiltrating macro-metastasis and disseminated multi-organ micro-metastases presenting as acute unilateral vestibular syndrome, due to the reawakening of a primary gastric signet ring cell carcinoma. Purpose – Our objective was to identify those pathophysiological steps that may explain the complex process of tumor reawakening, dissemination. The possible causes of vestibular asymmetry were also traced. A 56-year-old male patient’s interdisciplinary medical data had been retrospectively analyzed. Original clinical and pathological results have been collected and thoroughly reevaluated, then new histological staining and immunohistochemistry methods have been added to the diagnostic pool. During the autopsy the cerebrum and cerebellum was edematous. The apex of the left petrous bone was infiltrated and destructed by a tumor mass of 2x2 cm in size. Histological reexamination of the original gastric resection specimen slides revealed focal submucosal tumorous infiltration with a vascular invasion. By immunohistochemistry mainly single infiltrating tumor cells were observed with Cytokeratin 7 and Vimentin positivity and partial loss of E-cadherin staining. The subsequent histological examination of necropsy tissue specimens confirmed the disseminated, multi-organ microscopic tumorous invasion. Discussion – It has been recently reported that the expression of Vimentin and the loss of E-cadherin is significantly associated with advanced stage, lymph node metastasis, vascular and neural invasion and undifferentiated type with p<0.05 significance. As our patient was middle aged and had no immune-deficiency, the promoting factor of the reawakening of the primary GC malignant disease after a 9-year-long period of dormancy remained undiscovered. The organ-specific tropism explained by the “seed and soil” theory was unexpected, due to rare occurrence of gastric cancer to metastasize in the meninges given that only a minority of these cells would be capable of crossing the blood brain barrier. Patients with past malignancies and new onset of neurological symptoms should alert the physician to central nervous system involvement, and the appropriate, targeted diagnostic and therapeutic work-up should be established immediately. Targeted staining with specific antibodies is recommended. Recent studies on cell lines indicate that metformin strongly inhibits epithelial-mesenchymal transition of gastric cancer cells. Therefore, further studies need to be performed on cases positive for epithelial-mesenchymal transition.

Clinical Neuroscience

Atypical presentation of late-onset Sandhoff disease: a case report

SALAMON András , SZPISJAK László , ZÁDORI Dénes, LÉNÁRT István, MARÓTI Zoltán, KALMÁR Tibor , BRIERLEY M. H. Charlotte, DEEGAN B. Patrick , KLIVÉNYI Péter

Sandhoff disease is a rare type of hereditary (autosomal recessive) GM2-gangliosidosis, which is caused by mutation of the HEXB gene. Disruption of the β subunit of the hexosaminidase (Hex) enzyme affects the function of both the Hex-A and Hex-B isoforms. The severity and the age of onset of the disease (infantile or classic; juvenile; adult) depends on the residual activity of the enzyme. The late-onset form is characterized by diverse symptomatology, comprising motor neuron disease, ataxia, tremor, dystonia, psychiatric symptoms and neuropathy. A 36-year-old female patient has been presenting progressive, symmetrical lower limb weakness for 9 years. Detailed neurological examination revealed mild symmetrical weakness in the hip flexors without the involvement of other muscle groups. The patellar reflex was decreased on both sides. Laboratory tests showed no relevant alteration and routine electroencephalography and brain MRI were normal. Nerve conduction studies and electromyography revealed alterations corresponding to sensory neuropathy. Muscle biopsy demonstrated signs of mild neurogenic lesion. Her younger brother (32-year-old) was observed with similar symptoms. Detailed genetic study detected a known pathogenic missense mutation and a 15,088 base pair long known pathogenic deletion in the HEXB gene (NM_000521.4:c.1417G>A; NM_000521:c.-376-5836_669+1473del; double heterozygous state). Segregation analysis and hexosaminidase enzyme assay of the family further confirmed the diagnosis of late-onset Sandhoff disease. The purpose of this case report is to draw attention to the significance of late-onset Sandhoff disease amongst disorders presenting with proximal predominant symmetric lower limb muscle weakness in adulthood.

Clinical Neuroscience

A variant of Guillain-Barre syndrome after SARS-CoV-2 vaccination: AMSAN

TUTAR Kaya Nurhan, EYIGÜRBÜZ Tuğba, YILDIRIM Zerrin, KALE Nilufer

Introduction - Coronavirus disease 2019 (COVID-19) is a respiratory infection that has rapidly become a global pandemic and vaccines against SARS-CoV-2 have been developed with great success. In this article, we would like to present a patient who developed Guillain-Barré syndrome (GBS), which is a serious complication after receiving the inactive SARS-CoV-2 vaccine (CoronaVac). Case report – A 76-year-old male patient presented to the emergency department with nine days of progressive limb weakness. Two weeks prior to admission, he received the second dose of CoronaVac vaccine. Motor examination revealed decreased extremity strength with 3/5 in the lower extremities versus 4/5 in the upper extremities. Deep tendon reflexes were absent in all four extremities. Nerve conduction studies showed predominantly reduced amplitude in both motor and sensory nerves, consistent with AMSAN (acute motor and sensory axonal neuropathy). Conclusion - Clinicians should be aware of the neuro­logical complications or other side effects associated with COVID-19 vaccination so that early treatment can be an option.

Clinical Neuroscience

Acute transverse myelitis after inactivated COVID-19 vaccine

ERDEM Şimşek Nazan, DEMIRCI Seden, ÖZEL Tuğba , MAMADOVA Khalida, KARAALI Kamil , ÇELIK Tuğba Havva , USLU Ilgen Ferda, ÖZKAYNAK Sibel Sehür

Vaccines against the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) have been rapidly developed to prevent coronavirus disease 2019 (COVID-19) pandemic. There is increasing safety concerns regarding COVID-19 vaccines. We report a 78-year old woman who was presented with tetraparesis, paresthesias of bilateral upper extremities, and urinary retention of one-day duration. Three weeks before these symptoms, she was vaccinated with CoronaVAC vaccine (Sinovac Life Sciences, China). Spine magnetic resonance imaging showed longitudinally extensive transverse myelitis (TM) from the C1 to the T3 spinal cord segment. An extensive diagnostic workup was performed to exclude other possible causes of TM. We suggest that longitudinally extensive TM may be associated with COVID-19 vaccination in this case. To the best of our knowledge, this is the first report of longitudinally extensive TM developing after CoronaVac vaccination. Clinicians should be aware of neurological symptoms after vaccination of COVID-19.

Clinical Neuroscience

[Effective therapy in highly active pediatric multiple sclerosis ]

MERÔ Gabriella, MÓSER Judit, LIPTAI Zoltán, DIÓSZEGHY Péter, BESSENYEI Mónika, CSÉPÁNY Tünde

[Multiple sclerosis (MS) is typically a disease of young adults. Childhood MS can be defined in patients under 18 years of age, although some authors set the limit un­der the age of 16 formerly known as “early-onset multiple sclerosis” or “juvenile multiple sclerosis”, seen in 3-5% of all MS patients. Nowadays, owing to ever-evolving, better diagnostic tools and well-traced, strictly defined diagnostic criteria, childhood MS is showing an increasing incidence worldwide (0.05-2.85/100 000). MS is characterized by recurrent episodes of the central nervous system with demyelination separated in space and time. In childhood almost exclusively the relapsing-remitting (RR) type of MS occurs. Based on experience in adults, the goal in the pediatric population is also the early diagnosis, to initiate adequate DMT as soon as possible and to achieve symptom relief and good quality of life. Based on efficacy and safety studies in the adult population, inter­feron β-1a and glatiramer acetate were first approved by the FDA and EMA for the treatment of childhood MS also. The increased relapse rate and rapid progression of childhood MS and unfavorable therapeutic response to nearly 45% of the first DMT necessitated the testing of more effective and second-line drugs in the population under 18 years of age (PARADIGMS, CONNECT). Although natalizumab was reported to be effective and well-tolerated in highly active RRMS in childhood, evidence based studies were not yet available when our patients’ treatment started. In this article, we report on the successful treatment of three active RRMS patients with individually authorized off-label use of natalizumab.]